Macrophage activation syndrome
Macrophage activation syndrome | |
---|---|
Other names | MAS |
Specialty | Rheumatology |
Macrophage activation syndrome is a severe, potentially life-threatening, complication of several chronic rheumatic diseases of childhood. It occurs most commonly with
Signs and symptoms
The hallmark clinical and laboratory features include high fever,
Cause
In many cases a trigger is identified, often a viral infection, or a medication.
Diagnosis
A febrile patient with known or suspected SoJIA must be considered for macrophage activation if:
- Ferritin >684 ng/ml
and any 2 of the following:
- Hemoglobin <90g/L (in infants <4 weeks: <100g/L)
- Platelets <100 x 109/L
- Neutrophils <1.0 x 109/L
- Fasting triglycerides ≥3.0 mmol/L (i.e., ≥ 265 mg/dl)
- Fibrinogen ≤1.5 g/L[3]
In addition, other specific markers of macrophage activation (e.g. soluble CD163), and lymphocyte activation (e.g. soluble IL-2 receptor) can be helpful. NK cell function analysis may show depressed NK function, or, flow cytometry may show a depressed NK cell population. [4]
Treatment
The best treatment for MAS has not been firmly established. Most commonly used treatments include high-dose
Anakinra is also effective.[5]
See also
References
External links
- Macrophage Activation Syndrome, MedScape